Table 3

Pathological findings on examination of full-thickness rectal tissue from all patients with megarectum

Findingn (%)Notes
Melanosis0/33 (0)
Fibrosis30/35 (85.7)Focal submucosal only: 8
Submucosal and periplexus: 4
Submucosal and muscularis propria: 2
Submucosal, periplexus and muscularis propria: 10
Other combinations: 6
Elastosis19/33 (57.6)
1 site: 8
2 sites: 6
3 sites: 5
Submucosal only: 1*
Periplexus only: 2
Muscularis propria only: 4†
Serosa only: 1
Submucosa and muscularis propria: 2
Submucosa and periplexus: 1
Periplexus and muscularis propria: 1‡
Periplexus and serosa: 1
Muscularis propria and serosa: 1
Muscularis mucosae, submucosa and muscularis propria: 1
Submucosa, periplexus and muscularis propria: 2
Submucosa, muscularis propria and serosa: 2
Neuronal abnormalities6/35 (17.1)Degenerative or inflammatory neuropathy: 0
Hypoganglionosis: 2
Duplicated plexus: 3
Single ectopic ganglion: 1
Muscular abnormalitiesAbnormal muscle layers: 14/35 (40.0)Degenerative or inflammatory myopathy: 0
Submucosal metaplasia: 8
Duplication of circular muscle layer: 3
Partial duplication longitudinal muscle layer: 2
Grossly thinned: 1§
Muscle hypertrophy: 27/35 (77.1)Hypertrophy circular muscle layer only: 2
Hypertrophy both muscle layers: 25
Decreased interstitial cells of Cajal

6/27 (22.2)Qualitative assessment: only gross reductions reported
Polyglucosan bodies15/32 (46.9)Present on single section: 6
Present on multiple sections: 9
  • *Predominantly around the submucosal vessels.

  • †Includes one patient with elastosis confined to partial extra muscle layer.

  • ‡With granulomatous response to elastin fibres.

  • §Appearances suggest developmental abnormality.